Station 1.6: Pulmonary fibrosis Flashcards
Pulmonary fibrosis
Clinical signs
What are the clinical signs of Pulmonary fibrosis?
Pulmonary fibrosis
Examine this patient’s respiratory system, she has been complaining of progressive shortness of
breath.
* Clubbing, central cyanosis and tachypnoea
* Fine end‐inspiratory crackles (like Velcro® which do not change with coughing)
* Signs of associated autoimmune diseases, e.g. rheumatoid arthritis (hands), SLE and
systemic sclerosis (face and hands)
* Signs of treatment, e.g. Cushingoid from steroids
* Discoloured skin (grey) – amiodarone
* Unless there are any associated features then describe your findings as pulmonary
fibrosis, which is a clinical description pending further differentiation following
investigations
Discussion
How would you investigate Pulmonary fibrosis?
Pulmonary fibrosis
Examine this patient’s respiratory system, she has been complaining of progressive shortness of
breath.
Bloods:** ESR, rheumatoid factor and ANA
** CXR: reticulonodular changes; loss of definition of either heart border; small lungs
* ABG: type I respiratory failure
* Lung function tests:
⚬ FEV1
/FVC > 0.8 (restrictive)
⚬ Low TLC (small lungs)
* Reduced TLco and KCO
* Bronchoalveolar lavage: main indication is to exclude any infection prior to
immunosuppressants plus if lymphocytes > neutrophils indicate a better response to
steroids and a better prognosis (sarcoidosis)
* High-resolution CT scan: distribution helps with diagnosis; bibasal subpleural
honeycombing typical of UIP; widespread ground glass shadowing more likely to be
non‐specific interstitial pneumonia often associated with autoimmune disease; if apical
in distribution then think of sarcoidosis, ABPA, old TB, hypersensitivity pneumonitis,
Langerhan’s cell histiocytosis.
* Lung biopsy (associated morbidity ~7%)
Discussion
How would you treat Pulmonary fibrosis?
Pulmonary fibrosis
Examine this patient’s respiratory system, she has been complaining of progressive shortness of
breath.
* Immunosuppression if likely to be inflammatory; i.e. non‐specific interstitial
pneumonia e.g. steroids: combination of steroids and azathioprine no longer used
following results of PANTHER trial which showed increased morbidity on this
combination
* Pirfenidone (an antifibrotic agent) ‐ for UIP when FEV1
50–80% predicted (NICE
recommended)
* N‐acetyl cycsteine – free radical scavenger
* Single lung transplant
* NB: Beware single lung transplantation patient – unilateral fine crackles and
contralateral thoracotomy scar with normal breath sounds
Discussion
Prognosis for Pulmonary fibrosis?
Pulmonary fibrosis
Examine this patient’s respiratory system, she has been complaining of progressive shortness of
breath.
* Very variable: depends on aetiology
* Highly cellular with ground glass infiltrate – responds to immunosuppression: 80%
5‐year survival
* Honeycombing on CT – no response to immunosuppression: 80% 5‐year mortality
* There is an increased risk of bronchogenic carcinoma
Discussion
What are the causes of basal fibrosis?
Pulmonary fibrosis
Examine this patient’s respiratory system, she has been complaining of progressive shortness of
breath.
* Usual interstitial pneumonia (UIP)
* Asbestosis
* Connective tissue diseases
* Aspiration
